Metastatic Paraganglioma: A rare Endocrine Tumor with an atypical presentation

Abstract

Background: Paraganglioma, rare endocrine tumors, are considered like pheochromocytomas but they arise from the autonomic ganglia outside the adrenal medulla. We report a case of a male with paraganglioma who presented with paraplegia and with an extensive metastatic disease at presentation.

Case presentation: A 48-year-old male presented with paraplegia for one week in neurosurgery clinic. He had a history of weight loss and intermittent hypertension for 1 year which was not associated with any headache, sweating or palpitation. His history of note was a hypertensive spell during his cholecystectomy three years ago, but it was not worked up further. His MRI spine showed multiple lytic lesions in the thoracic and lumber spine with a paraspinous mass and multiple enlarged lymph nodes in the para-spinal and para-aortic location. His PET scan showed extensive disease with very high SUVs. A CT guided biopsy of Left paraspinal mass suggested paraganglioma. At this stage he was referred to the Endocrinology clinic. His plasma metanephrines levels were done which showed metanephrines 5840pg/ml (ref. range 0-190). Since the neurosurgeon decided for surgery, he was started on alpha blockers (Doxazocin 4 mg twice daily) followed by Beta blockers (Atenolol 75 mg once daily) to control blood pressure and prevent any Hypertensive crisis during surgery. He underwent D4 laminectomy and spinal decompression. After surgery his paraplegia improved to some extent. His I131 MIBG (Metaiodobenzylguanidine) scan was negative so this could not be a possible therapeutic option. His presentation suggests a very high possibility of SDHB mutation of paragangliomas. We have discussed the option of genetic testing with the family, but this is not available in Pakistan, so it could not be done till now. The three siblings of the patients were screened with the urine metanephrines, and all came out to be negative. The patient has been started on Chemotherapy with a VAD (Vincristine, Adriamycin, Doxorubicin) regimen and has experienced a slight improvement in his clinical condition.

Conclusion: Any patient with an increased pressor response to surgery or a diagnostic procedure must be screened for paraganglioma pheochromocytoma syndrome.